Reversed Potts Shunt and Venopulmonary Extracorporeal Membrane Oxygenation Support in Sustained Pulmonary Hypertension Crisis

Background

Infantile idiopathic pulmonary arterial hypertension complicated by recurrent episodes of pulmonary hypertensive crisis is an extremely rare and critical condition with high mortality.

Case Summary

We present the case of a 5-month-old infant with a pulmonary hypertension crisis refractory to medical therapy, who underwent successful treatment with an emergency reversed Potts shunt (RPS), along with postoperative venopulmonary extracorporeal membrane oxygenation (V-P ECMO) support.

Discussion

From an imaging perspective, the mechanism by which RPS improves symptoms in patient with sustained pulmonary hypertension crises is explained. Additionally, this is the first reported case of successful postoperative use of V-P ECMO support after RPS.

Take-Home Messages

It is important to recognize the importance of RPS and V-P ECMO in the treatment of pulmonary hypertension crises. By comparing pre- and postoperative imaging data, the mechanism by which RPS improves cardiac function is better understood.